Department of Pediatric Surgery, Virgen del Rocio Children's Hospital, Sevilla, Spain.
Department of Pediatric Oncology, Virgen del Rocio Children's Hospital, Sevilla, Spain.
Dermatol Pract Concept. 2015 Jan 30;5(1):91-3. doi: 10.5826/dpc.050118. eCollection 2015 Jan.
Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor that may be complicated by Kasabach-Merritt phenomenon (KMP), a profound thrombocytopenia resulting from platelet trapping within a vascular tumor, either KHE or tufted angioma (TA). Typical features also include low fibrinogen and elevated D-dimers. It is well known that KMP is not caused by infantile hemangiomas. Management of vascular tumors complicated by KMP is challenging, and it is common for referral centers to receive patients in critical medical condition after multimodality treatment failure of vascular anomalies. Our aim is to communicate the importance of early diagnosis and treatment of KHE associated with KMP. A full-term male newborn with KHE complicated by KMP is reported. Treatment with vincristine, aspirin and ticlopidine normalized the coagulation parameters within one week, requiring a total of six doses of vincristine, seven months of ticlopidine and 17 months of aspirin. Early diagnosis and treatment of KHE complicated by KMP may allow the administration of fewer doses of vincristine and avoid the use of corticosteroids.
卡波西样血管内皮细胞瘤（KHE）是一种局部侵袭性血管肿瘤，可能并发卡-梅现象（KMP），这是一种由于血小板在血管肿瘤内被捕获而导致的严重血小板减少症，这种血管肿瘤可以是 KHE 或丛状血管瘤（TA）。其典型特征还包括低纤维蛋白原和升高的 D-二聚体。众所周知，KMP 不是由婴儿血管瘤引起的。并发 KMP 的血管肿瘤的治疗具有挑战性，转诊中心经常在血管异常的多模式治疗失败后接收处于危急医疗状况的患者。我们的目的是强调早期诊断和治疗与 KMP 相关的 KHE 的重要性。报告了一例伴 KMP 的足月男性新生儿 KHE。长春新碱、阿司匹林和噻氯匹定治疗在一周内使凝血参数正常化，总共需要 6 剂长春新碱、7 个月的噻氯匹定和 17 个月的阿司匹林。早期诊断和治疗伴 KMP 的 KHE 可能允许使用更少剂量的长春新碱，并避免使用皮质类固醇。
